Pediatric Idiopathic Scoliosis

Author(s): Yuxi Chen, MD, Matthew Oglesby, MD, and Nan Wang, MD

Originally published:5/29/2018

Last updated:

1. Disease/Disorder: Pediatric Idiopathic Scoliosis

Definition

Scoliosis is a 3-dimensional deformity of the spine and trunk. It is identified by a lateral curvature of the spine with a Cobb angle >10 degrees as measured on an anterior-posterior standing radiograph. 80% of all pediatric scoliosis is termed “idiopathic” or of unknown etiology.1,2

Etiology

Although several factors have been proposed, including genetics; connective tissue abnormalities; and associated neurological, muscular, and skeletal disorders, its exact etiology is still unclear. It remains a diagnosis of exclusion and its evaluation is consistent with a normal neurological exam and lack of anomalous vertebrae on imaging studies.3

Epidemiology including risk factors and primary prevention

The prevalence of idiopathic scoliosis (IS) in the general population is approximately 1.9%- 3% for curves >10 degrees and 0.3% for curves >20 degrees. 1,2 It is stratified into three sub-types:

  • Infantile (birth to 3 years):  <1% of diagnoses. M:F (3:2).
  • Juvenile (3 to 9 years): 12 – 21% of diagnoses. F:M (1.6-4.4:1.0); ratio increases with increasing age of onset.
  • Adolescent (10 to 18 years) (AIS): 80 – 90% of diagnoses. F:M (5:1)

 

  • Cobb angle ≥10°: 3%; F:M -> 2.4:1
  • Cobb angle ≥20°: 0.5%; F:M -> 5.4:1
  • Cobb angle ≥30°: 0.3%; F:M -> 10:1
  • Cobb angle ≥40°: ≤0.1%

F = females; M = males.

Risk factors: family history; joint hypermobility; growth-related factors (increased height and growth velocity); low body mass index (BMI); and female with delayed puberty and late menarche.4

Patho-anatomy/physiology

Spinal stability is derived from bone, connective tissue, muscle, and their interaction with the nervous system. Dysfunction in any of these elements leads to imbalance and, ultimately, to deformity. This deformity may manifest as a curvature in the anterior-posterior plane, angulation in the sagittal plane, and/or a rotation in the transverse plane.

IS is a complex, multifactorial condition. The pathogenesis remains limited.

Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time):

Infantile: Most tend to spontaneously resolve.

Juvenile: Slow progression until about 10 years of age when curve progression is more rapid, coinciding with the period of accelerated spine growth; more likely to have curves that progress to severe deformity and less likely to respond to nonsurgical treatment such as bracing.

Adolescent: The risk of progression depends on skeletal maturity, sex, and curve magnitude. Patients with curves >20 degrees who are skeletally immature are at a greater risk for curve progression. 1 Potential for progression is much lower after the completion of spinal growth. However, if curve >50 degrees, scoliosis is going to progress in adulthood and cause health problems and reduction of quality of life.2

Specific secondary or associated conditions and complications

Severe scoliosis can lead to skin breakdown (ribs overlapping pelvis), poor seating biomechanics, aesthetic concerns and restrictive lung disease. An increased risk of morbidity and mortality may be seen in younger patients (<10 years of age) who present with a compromised pulmonary status.

Spinal deformity may also lead patient experiencing chronic spinal pain in adulthood.2

2. Essentials of Assessment

History

Keys in the history include age of onset and history of spinal deformity progression. The usual presenting complaint is chest wall or back asymmetry, though about 25% will present with non-specific pain.

Rapid development of a severe curve suggests a nonidiopathic type of scoliosis. Weakness, sensory changes, gait abnormalities, radiating pain, bowel or bladder control changes and other neurologic symptoms suggest intraspinal pathology such as syringomyelia, tethered cord, tumor, etc.5

History of difficulty breathing may suggest pulmonary compromise and is an indication for further evaluation.

Physical examination

Monitor for peak growth velocity by assessing pubertal development and plotting heights on a growth chart.

Look for asymmetry of limb length (curves caused by a leg-length discrepancy should disappear with patients sitting), shoulder height and scapular positioning, pelvic tilt, and chest cage deviation. Check for joint deformity and excessive laxity.

Perform Adam’s forward-bend test by having the patient bend forward at the waist with knees straight. Asymmetry is evidenced by rib prominence and/or lumbar paraspinal elevation.

A scoliometer measures rotational deformity on the Adam’s forward bending test, and a trunk rotation of >7 degrees is an indication for referral. Modern scoliometers are now readily available as applications for smart phones.

Neurologic exam includes motor strength in all 4 limbs, sensation, reflexes, gait, tandem walk, and abdominal reflex.

Visually inspect for skin markings suggestive of other causes such as sacral dimple, hair patches, hemangiomas, and café au lait spots.

Clinical functional assessment: mobility, self care cognition/behave or/affective state

Activities of daily living (ADL), Functional Independence Measure (FIM), and WeeFIM measures can be helpful in assessing function (self-care, sitting balance, breathing, feeding, transfers, mobility, ambulation, bowel and bladder management, etc.). Cognition is not affected by scoliosis. Some patients may become power wheelchair users due to mechanical or cardiopulmonary limitations.

Laboratory studies

Unnecessary except in work-up for metabolic conditions.

Imaging

Initial radiographic evaluation of patients is with a standing posterior-anterior (PA) and lateral radiograph of the whole spine. The curve magnitude is evaluated using the Cobb method of measuring the angular deformity from the upper end vertebrae to the lower end vertebrae. On the lateral radiograph, there is generally an apical hypokyphosis associated with idiopathic scoliosis.1

Examine for features of skeletal maturity, including the Risser sign (maturity of iliac crest apophysis) and open versus closed triradiate cartilage of the acetabulum.

Routine MRI not indicated.  Consider MRI for congenital scoliosis, abnormal neurologic exam, musculoskeletal deformity such as cavus feet, signs associated with underlying neural tube defect, or left thoracic curvature with rapid progression.

Supplemental assessment tools

Obtain pulmonary function tests for those with severe curves causing restrictive lung disease. Moderate curves can benefit from baseline PFTs due to risk of pulmonary compromise. A CT scan can evaluate 3-dimensioinal lung volume in young patients who may not be able to comply with pulmonary function testing.

Supine bending and stretch radiographs can be used to assess the flexibility of the curve.

Early prediction of outcomes

Factor determinants of a higher risk of scoliosis progression: positive family history, laxity of skin and joints (connective tissue defect), flattening of physiological thoracic kyphosis (impedes efficient bracing), angle of trunk rotation exceeding 10, and growth spurt.2

Environmental

There are no identifiable environmental factors that influence the condition of pediatric idiopathic scoliosis.

Social role and social support system

Psychosocial history is important in assessing adolescents with scoliosis, as patients are often not happy with the cosmetic deformity. It is important to inquire in this regard and consult a rehabilitation psychologist or social worker to address the emotional concerns of the patient and family, if needed.

Professional issues

(Identify and/or consider issues relevant to this topic in the areas of ethics, quality of life, professionalism and safety.)

Adolescence is a phase of development during which establishment of self-respect and self-confidence occurs. Although idiopathic scoliosis is usually asymptomatic, it may make the process challenging and difficult. It may progress to back pain, respiratory problems, and changes in physical appearance, thus negatively impacting quality of life.6

Screening for AIS is controversial; however, the Scoliosis Research Society, the Pediatric Orthopedic Society of North America, the American Academy of Orthopedic Surgeons, and the American Academy of Pediatrics all agree that girls should be screened twice, at 10 and 12 years of age, whereas boys should be screened once at 13 or 14 years of age.1,2,7

3. Rehabilitation Management and Treatments

Available or current treatment guidelines

2016 guidelines from The International Scientific Society on Scoliosis Orthopaedic and Rehabilitation Treatment (SOSORT) provide current evidence and recommendations on conservative treatment ( braces and exercise) of idiopathic scoliosis.2

The goal of scoliosis treatment is to prevent further progression of the curvature with a goal of <50 degrees at skeletal maturity. 1 Current management of scoliosis includes observation, bracing, surgery, and the choice of treatment is based on the location and type of the curve, the degree of curvature, and bone maturity.1

At different disease stages.

In patients with Cobb angle <30 degrees and Risser grade 0 to 2, observation alone is recommended. 7  In patients with Cobb angle 30 to 40 degrees and Risser grade 0 to 2, bracing is suggested and should be continued until skeletal maturity.8

Weinstein et al. demonstrated in the BRAIST trial (NEJM, 2013) that bracing significantly decreases the progression of high-risk curves to the threshold for surgery (50 degrees), and longer hours of brace wearing increases the efficacy of bracing, with 13 hours or more being adequate. 2,8  Other controlled prospective studies also suggested a benefit of  bracing on slowing curvature progression.7

Thoracolumbosacral orthosis (TLSO) is the  most commonly used rigid brace.  Other bracing options include night-wearing braces (Charleston and the Providence), nonrigid brace (SpineCor), and more invasive brace as Milwaukee with under chin extension.  9, 10, 11 A cast is considered a standard first approach in infantile scoliosis, followed by rigid brace.2

There is strong evidence supporting the use of scoliosis specific PT and exercise for AIS. Therapy may be used alone or in conjunction with braces in patients with AIS with curves of less than 45°.2

Surgical treatment is reserved for Cobb angle >45 degrees in skeletally immature patients and Cobb angle >50 degrees in skeletally mature patients. 1 Surgery to correct scoliosis involves spinal fusion, and posterior spinal fusion with instrumentation is the most common surgical procedure for adolescent idiopathic scoliosis. 13

Coordination of care

(Recommend useful team approaches or models of care relevant to this topic.)

Interdisciplinary discussion among pediatrician, physiatrist, and orthopedic surgeon should be held to determine optimal treatment. In complex cases, this is particularly important to prevent progression of the disease.

Patient & family education

The patient and their family should be educated on the nature and progression of   idiopathic scoliosis. They should be aware that treatment is individualized, based on the disease severity and potential for growth. Different treatment options along with their risks and benefits should be thoroughly explained.

Measurement of Treatment Outcomes including those that are impairment-based, activity participation-based and environmentally-based.

Several factors have been studied to examine the treatment outcome of AIS. One of them is curve progression. 1 It is estimated that the curve tends to progress one degree per year after skeletal maturity if it is greater than 50 degrees at the end of growth, in contrast to no curve progression if the curve is less than thirty degrees at the end of growth. 1,8

Other measurements of the treatment outcome include back pain, self-image and quality of life. A series of instruments (questionnaires) for post spinal surgery patients have been proposed to evaluate quality of life.2

Translation into Practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills

Early diagnosis and monitoring is vital in the treatment of idiopathic scoliosis. The degree of curvature and skeletal maturity will dictate treatment which will range from observation to bracing to surgery.

Prospective evidence now supports the benefit of bracing and scoliosis specific PT and exercise in the treatment of AIS. 2,10

Cutting edge/emerging and unique concepts and practice

The dynamic surface topography (DST) is a rasterstereography based on imaging system designed to evaluate spinal deformity, providing radiation-free imaging of the position, rotation, and shape of the trunk during the gait cycle. Imaging of DST is based on optical acquisition of the whole torso surface. The test aims to decrease the cumulative exposure to X-ray radiation. It is useful in patient monitoring as an alternative to radiography, without diminishing the standard of care.2

Gaps in the evidence-based knowledge

Studies on long-term outcomes such as pulmonary disorders, disability, back pain, psychological effects, cosmetic issues, and quality of life are needed and should stratify results by degree of spinal curvature at diagnosis and at skeletal maturity.9

There is also need for developing the questionnaire specifically for measuring quality of life  (QoL) in patients treated conservatively, allowing comparisons of QoL between patients treated differently.2

References

  1. El-Hawary R, Chukwunyerenwa C. Update on Evaluation and Treatment of Scoliosis. Pediatric Clinics of North America. Vol 61, issue 6, Dec 2014. Pages 1223-1241.
  2. Negrini S, Donzelli S, Aulisa AG,et al. 2016 SOSORT guidelines: Orthopaedic and Rehabilitation Treatment of Idiopathic Scoliosis during Growth. Scoliosis Spinal Disord. 2018; 13:3. Epub 2018 Jan 10.
  3. Zaydman AM, Strokova EL, Kiseleva EV, et al. A New Look at Etiological Factors of Idiopathic Scoliosis: Neural Crest Cells. International Journal of Medical Sciences. 2018;15(5):436-446. doi:10.7150/ijms.22894.
  4. Fadzan M, Bettany-Saltikov J. Etiological Theories of Adolescent Idiopathic Scoliosis: Past and Present. Open Orthop J. 2017 Dec 29;11:1466-1489. doi:10.2174/1874325001711011466. eCollection 2017.
  5. Newton PO, Wenger DR.Idiopathic scoliosis.In: Morrissy RT,Weinstein SL, eds.Lovell and Winters Pediatric Orthopaedics. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2006:695-761.
  6. Meng Z-D, Li T-P, Xie X-H, Luo C, Lian X-Y, Wang Z-Y. Quality of life in adolescent patients with idiopathic scoliosis after brace treatment: A meta-analysis. Sharma. VP, ed. Medicine. 2017;96(19):e6828. doi:10.1097/MD.0000000000006828.
  7. Screening for Adolescent Idiopathic Scoliosis: US Preventive Services Task Force Recommendation Statement.JAMA. 2018 Jan 9;319(2):165-172. doi: 10.1001/jama.2017.19342.
  8. Weinstein SL, Dolan LA, Wright JG, Dobbs MB. Effects of bracing in adolescents with idiopathic scoliosis. N Engl J Med. 2013 Oct 17; 369(16):1512-21.
  9. Screening for Adolescent Idiopathic Scoliosis: US Preventive Services Task Force Recommendation Statement.JAMA. 2018 Jan 9;319(2):165-172. doi: 10.1001/jama.2017.19342.
  10. Gammon SR, Mehlman CT, Chan W, et al. A comparison of thoracolumbosacral orthoses and SpineCor treatment of adolescent idiopathic scoliosis patients using the Scoliosis Research Society standardized criteria. J Pediatr Orthop 2010; 30:531.
  11. Janicki JA, Poe-Kochert C, Armstrong DG, Thompson GH. A comparison of the thoracolumbosacral orthoses and providence orthosis in the treatment of adolescent idiopathic scoliosis: results using the new SRS inclusion and assessment criteria for bracing studies. J Pediatr Orthop 2007; 27:369.
  12. Lonstein JE, Winter RB. The Milwaukee brace for the treatment of adolescent idiopathic scoliosis. A review of one thousand and twenty patients. J Bone Joint Surg Am 1994; 76:1207.
  13. Geck MJ, Rinella A, Hawthorne D, et al. Comparison of surgical treatment in Lenke 5C adolescent idiopathic scoliosis: anterior dual rod versus posterior pedicle fixation surgery: a comparison of two practices. Spine (Phila Pa 1976) 2009; 34:1942.

Author Disclosure

Yuxi Chen, MD
Ipsen: Research Grants – Principle investigator; Honorarium – Advisory
Merz: Honorarium – Advisory

Matthew Oglesby, MD
Nothing to Disclose

Nan Wang, MD
Nothing to Disclose

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